6 research outputs found
Pediatric rheumatology in Africa: thriving amidst challenges
No full textBackground: Pediatric Rheumatology is an orphan specialty in Africa which is gradually gaining importance across the continent.
Main body: This commentary discusses the current state of affairs in the sphere of Pediatric Rheumatology across Africa and offers practical strategies to navigate the challenges encountered in research, models of care, education and training. We outline the establishment, opportunities of growth and achievements of the Pediatric Society of the African League Against Rheumatism (PAFLAR).
Conclusion: This commentary lays the foundation for establishment of a formidable framework and development of partnerships for the prosperity of Pediatric Rheumatology in Africa and beyond
COVID-19 and the practice of rheumatology in Africa: big changes to services from the shockwave of a pandemic
No full textThe onset of the COVID-19 pandemic has led to far-reaching changes in the delivery of healthcare services across Africa. A number of drugs used in the management of rheumatic diseases have been touted to have roles to play in the treatment and/or exacerbation of COVID-19 symptoms and this has resulted in significant changes in the practice of rheumatology. The global rheumatology community has risen to this challenge by demonstrating collaborative partnership, resulting in the establishment of the global rheumatology registry to collect data on rheumatic patients infected with COVID-19.1 In view of the study by Gianfrancesco et al ,2 an online survey consisting of 40 practice and experience questions ((online supplementary file 1) and (online supplementary file 2)) was created by the COVID-19 African Rheumatology Study Group which was formed through the network of the African League of Associations for Rheumatology (AFLAR). The aim of the study was to identify the changes in rheumatology practice and patient behaviour, as well as to highlight key concerns of rheumatologists across Africa resulting from the ongoing COVID-19 pandemic
Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East
No full textJuvenile Idiopathic Arthritis (JIA) is a group of chronic heterogenous disorders that manifests as joint inflammation in patients aged <16 years. Globally, approximately 3 million children and young adults are suffering from JIA with prevalence rates consistently higher in girls. The region of Africa and Middle East constitute a diverse group of ethnicities, socioeconomic conditions, and climates which influence the prevalence of JIA. There are only a few studies published on epidemiology of JIA in the region. There is an evident paucity of adequate and latest data from the region. This review summarizes the available data on the prevalence of JIA and its subtypes in Africa and Middle East and discusses unmet needs for patients in this region. A total of 8 journal publications were identified concerning epidemiology and 42 articles describing JIA subtypes from Africa and Middle East were included. The prevalence of JIA in Africa and Middle East was observed to be towards the lower range of the global estimate. We observed that the most prevalent subtype in the region was oligoarticular arthritis. The incidence of uveitis and anti-nuclear antibody (ANA) positivity were found to be lower as compared to the incidence from other regions. There is a huge unmet medical need in the region for reliable epidemiological data, disease awareness, having regional and local treatment guidelines and timely diagnosis. Paucity of the pediatric rheumatologists and economic disparities also contribute to the challenges regarding the management of JIA
Worldwide evaluation of Clinical Practice Strategies (CliPS) for lung involvement in Still's disease within the JIR-CliPS network: a COST action
No full textInternational multidisciplinary consensus on the definition and clinical approach for monogenic inflammatory immune dysregulation disorders
No full textAbstract Objective To achieve consensus on the definition and clinical approach of Monogenic Inflammatory Immune Dysregulation Disorders (MIIDDs), a collective term for rare conditions marked by inflammation, immune dysregulation, and infection susceptibility. These consensus guidelines specifically apply to pathogenic (or likely pathogenic) gene mutations affecting both innate and adaptive immunity, excluding variants of unknown significance (VUS). Methods A multi-step, evidence-based, multidisciplinary consensus process was employed, consisting of: (1) a systematic literature review across four electronic databases (Cochrane Library, Web of Science, Scopus, and MEDLINE via PubMed), updated through December 31, 2024; (2) a pre-Delphi electronic survey completed by 95 international adult and pediatric immunologists and rheumatologists; and (3) a modified online Delphi process with an international multidisciplinary expert panel, where statements were iteratively analyzed and refined until achieving consensus (≥ 80% agreement among panelists). Results Fifteen experts from 12 countries participated in two rounds of the Delphi process, resulting in the development of eight overarching principles and 10 consensus statements. These were categorized into five domains: (1) definitions and conceptual framework, (2) diagnostic and monitoring considerations, (3) treatment and therapeutic strategies, (4) multidisciplinary and collaborative care, and (5) patient education and support. Conclusion This consensus defines MIIDDs and provides a structured clinical framework to streamline research efforts and improve patient outcomes
Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
No full textObjective: To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice. Methods: A systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure. Results: Fifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS. Conclusion: High-dose GCs together with IL-1 and IFN gamma inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS. However, the global level of evidence on MAS treatment, especially in other conditions, is still poor and requires standardized studies to be confirme
